Abstract

Coenzyme Q10 (CoQ10) plays a key role as an electron carrier in the mitochondrial respiratory chain and as a cellular antioxidant molecule. A deficit in CoQ10 status may contribute to disease pathophysiology by causing a failure mitochondrial energy metabolism as well as compromising cellular antioxidant capacity. This chapter outlines the analytical methods used for determining cellular CoQ10 status using high-pressure liquid chromatography with ultraviolet (HPLC-UV) detection. In addition, we present a pharmacological procedure for establishing a human neuronal cell model of CoQ10 deficiency, for use in research studies.